ABSTRACT
With improved longevity in people with cystic fibrosis (pwCF), some manifestations of CF are increasingly seen. Some complications may result, at least in part, from deficiency of the cystic fibrosis transmembrane conductance regulator (CFTR) channel, while others result from treatments of known CF manifestations. Multiple factors, including CF-related diabetes and treatment with aminoglycosides, result in an increased prevalence of renal failure and auditory and vestibular dysfunction in pwCF. Increased inflammation elevates the risk of vasculitis and arthritis among pwCF, with an increased prevalence of serum autoantibodies. Venous thromboembolism is increasingly seen among children and adults with CF, especially those with endovascular catheters. Finally, increasing rates of overweight and obesity, dyslipidaemia, and endothelial dysfunction among pwCF elevate the risk of cardiovascular events. With the introduction of highly effective CFTR modulators (HEMT), prevention of some of these complications is likely needed. It is unclear, however, whether increased cardiovascular complications resulting from increasing rates of dyslipidaemia, hypertension, and obesity in pwCF with pre-existing inflammation commencing HEMT will be evident in the near future. Recognizing the less common, multifactorial manifestations and complications in CF is important for early detection and prevention of morbidity.
